Immunophenotypic but Not Genetic Changes Reclassify the Majority of Relapsed/Refractory Pediatric Cases of Early T-Cell Precursor Acute Lymphoblastic Leukemia.

Early T-cell precursor acute lymphoblastic leukemia (ETP-ALL) develops from very early cells with the potential for both T-cell and myeloid differentiation. The ambiguous nature of leukemic blasts in ETP-ALL may lead to immunophenotypic alterations at relapse. Here, we address immunophenotypic alterations and related classification issues, as well as genetic features of relapsed pediatric ETP-ALL.

Laboratory characterization of the pediatric B/T subtype of mixed-phenotype acute leukemia: Report of a case series.

Objectives: Mixed-phenotype acute leukemia (MPAL) is a rare disease associated with difficulties in the correct lineage assignment of leukemic cells. One of the least common subtypes within this category is characterized by the simultaneous presence of B- and T-lineage-defining antigens. Each case of suspected B/T MPAL should be considered in light of all available laboratory and clinical data to avoid misdiagnosis.

Platelets provide robustness of spatial blood coagulation to the variation of initial conditions.

Activated platelets provide phospholipid surface and secrete coagulation factors, enhancing blood clotting. We investigated the role of platelets in the regulation of blood coagulation spatial dynamics. We activated blood clotting with tissue factor-bearing (TF) surface in platelet-rich plasma (PRP) or platelet-free plasma (PFP).

Recognizing Minor Leukemic Populations with Monocytic Features in Mixed-Phenotype Acute Leukemia by Flow Cell Sorting Followed by Cytogenetic and Molecular Studies: Report of Five Exemplary Cases.

Mixed-phenotype acute leukemia (MPAL), a rare and heterogeneous category of acute leukemia, is characterized by cross-lineage antigen expression. Leukemic blasts in MPAL can be represented either by one population with multiple markers of different lineages or by several single-lineage populations. In some cases, a major blast population may coexist with a smaller population that has minor immunophenotypic abnormalities and may be missed even by an experienced pathologist.

Correlation of the surface expression of thymic stromal lymphopoietin receptor with the presence of CRLF2 gene rearrangements in children with B-lineage acute lymphoblastic leukemia.

Introduction: In this study, we aimed to compare the immunophenotype of tumor cells in children with B-cell precursor acute lymphoblastic leukemia (BCP-ALL) harboring rearrangements of the CRLF2 gene with that in children without such aberrations with a specific focus on the surface expression of the related protein thymic stromal lymphopoietin receptor (TSLPR).

Methods: We examined bone marrow samples from 46 patients with primary BCP-ALL who had CRLF2 rearrangements detected by FISH (CRLF2(+) cohort). A total of 140 consecutive patients with intact CRLF2 were included in a control CRLF2(-) cohort.

Legume NCRs and nodule-specific defensins of actinorhizal plants-Do they share a common origin?

The actinorhizal plant Datisca glomerata (Datiscaceae, Cucurbitales) establishes a root nodule symbiosis with actinobacteria from the earliest branching symbiotic Frankia clade. A subfamily of a gene family encoding nodule-specific defensin-like cysteine-rich peptides is highly expressed in D. glomerata nodules.

Total white blood cell counts but not HL ratio changes in the transition from post-juvenile molt to autumn migration in the first-year Eurasian blackcap (Sylvia atricapilla).

Theoretically, seasonal changes in immune functioning in animals are shaped by the trade-off between a probability of encountering pathogens and availability of resources. We used leukocyte profile (absolute and relative leukocyte counts) as a simple measure of immune system condition to study how it changes during the transition from postjuvenile molt to autumn migration in a free-living migratory songbird, the Eurasian blackcap (Sylvia atricapilla). We observed the higher white blood cells (WBC) and lymphocyte counts in molting birds compared to migrating individuals, but we did not find differences in heterophils and ratio of heterophils to lymphocytes (HL ratio).

Efficacy and safety of ruxolitinib in ineffective erythropoiesis suppression as a pretransplantation treatment for pediatric patients with beta-thalassemia major.

Background: Ineffective erythropoiesis (IE) is the most prominent feature of transfusion-dependent beta-thalassemia (TDT), which leads to extramedullary hemopoiesis. The rejection rate in allogeneic hematopoietic stem cell transplantation (HSCT) is high in heavily transfused patients with TDT accompanied by prominent IE. Therefore, a pretransplantation treatment bridging to HSCT is often used to reduce allosensitization and IE.

[A case report of familial dyskeratosis congenital. Case report].

Dyskeratosis congenita (DC) is a hereditary syndrome of bone marrow failure, which develops because of telomeres defects and combines with cancer predisposition. Its classical clinical features are skin pigmentation, nail dystrophy, oral leukoplakia (skin-mucosa triad). The goal is to describe the algorithm of diagnosis, clinical specificities of DC and specific treatment for cases of DC in one family.

Additional flow cytometric studies for differential diagnosis between Burkitt lymphoma/leukemia and B-cell precursor acute lymphoblastic leukemia.

The differentiation between Burkitt lymphoma/leukemia (BL) and B-cell precursor acute lymphoblastic leukemia (BCP-ALL) is sometimes complicated. Laboratory findings that favor BL (e.g.

Multiple bilateral hip fractures in a patient with dyskeratosis congenita caused by a novel mutation in the PARN gene.

We report a case of a young male patient with clinical signs of dyskeratosis congenita who presented with multiple bilateral low-traumatic hip fractures. Whole exome sequencing (WES) showed a previously unreported mutation in the poly(A)-specific ribonuclease (PARN) gene. Zoledronic acid 5 mg over 3 years was effective at preventing further fractures.

Recombinant Spidroin Films Attenuate Individual Markers of Glucose Induced Aging in NIH 3T3 Fibroblasts.

The effect of bioresorbable materials on aging in cultured mouse NIH 3T3 fibroblasts treated with elevated glucose concentration was investigated. The cells were grown on films produced from the silkworm fibroin and rS1/9, a recombinant analog of Nephila clavipes spidroin 1. Exposure to 50 mM glucose of the cells grown on uncoated glass support resulted in the cell growth retardation.

Accumulation of and Response to Auxins in Roots and Nodules of the Actinorhizal Plant Compared to the Model Legume .

Actinorhizal nodules are structurally different from legume nodules and show a greater similarity to lateral roots. Because of the important role of auxins in lateral root and nodule formation, auxin profiles were examined in roots and nodules of the actinorhizal species and the model legume . The auxin response in roots and nodules of both species was analyzed in transgenic root systems expressing a beta-glucuronidase gene under control of the synthetic auxin-responsive promoter .

[The concept of patient-oriented approach as a key tool of social economic development].

The article considers the need for a new model of health care. In the provision of patient care, individual needs and desired outcomes are the driving force behind all decisions in health and quality measurement. Medical care should correspond to individual preferences, needs and values of patients and take into account patient's wishes when making clinical decisions.

Heterogeneity of childhood acute leukemia with mature B-cell immunophenotype.

Background: Flow cytometry (FCM) plays a crucial role in the differential diagnosis of Burkitt lymphoma/leukemia (BL) and B-cell precursor acute lymphoblastic leukemia (BCP-ALL). The presence of surface IgM (sIgM) alone or with light chain restriction indicates a mature blast phenotype (BIV by EGIL) and is usually observed in BL. However, sIgM expression could also be detected in transitional BCP-ALL cases.

Bleeding tendency and platelet function during treatment with romiplostim in children with severe immune thrombocytopenic purpura.

It has been suggested that platelet function in chronic immune thrombocytopenic purpura (ITP) may be abnormal. Thrombopoietin mimetics used for treatment can affect it, but the data remain limited. We investigated platelet function of 20 children diagnosed with severe ITP (aged 1-16 years, 12 females and eight males).

Hysteresis-like binding of coagulation factors X/Xa to procoagulant activated platelets and phospholipids results from multistep association and membrane-dependent multimerization.

Binding of coagulation factors X (fX) and Xa (fXa) to activated platelets is required for the formation of membrane-dependent enzymatic complexes of intrinsic tenase and prothrombinase. We carried out an in-depth characterization of fX/fXa binding to phospholipids and gel-filtered, thrombin-activated platelets. Flow cytometry, surface plasmon resonance, and computational modeling were used to investigate interactions of fX/fXa with the membranes.

Candidatus Frankia Datiscae Dg1, the Actinobacterial Microsymbiont of Datisca glomerata, Expresses the Canonical nod Genes nodABC in Symbiosis with Its Host Plant.

Frankia strains are nitrogen-fixing soil actinobacteria that can form root symbioses with actinorhizal plants. Phylogenetically, symbiotic frankiae can be divided into three clusters, and this division also corresponds to host specificity groups. The strains of cluster II which form symbioses with actinorhizal Rosales and Cucurbitales, thus displaying a broad host range, show suprisingly low genetic diversity and to date can not be cultured.

[Organization and epidemiological surveillance of Western Nile fever in case of the Volgograd Region].

Western Nile fever (WNF) is an arboviral infection of the most relevance to the Volgograd Region. The observed decreasing incidence of WNF in 2001-2006, 2008, and 2009 and a decline in the number of its severe forms are only suggestive of the temporary regression of the epidemic process in the Volgograd Region. There are prerequisites and forerunners for a complication of the epidemiological Western Nile fever situation in the Volgograd Region.

[Epidemiological risk of introduction of dangerous and exotic infectious diseases on the territory of the XXII Olympic Winter Games and XI Paralympic Winter Games 2014 in Sochi].

To assess the epidemiological risk of introduction of serious infectious diseases in the pre-Olympic period defined list of dangerous and exotic infections and held assessment of potential danger threatening. Initial external information to assess the potential risk of skidding were reports, forecasts, posted on the official websites. The risk of skidding and epidemiological complications conditionally designated as high, moderate and minimal risk importation of measles virus-Rate was considered as high.

[Operation and interaction peculiarities of diagnostic laboratories involved in providing protection from infectious diseases during the XXII Olympic Winter Games and XI Paralympic Winter Games 2014 in Sochi].

The experience of the organization and functioning of the laboratory network during the XXII Olympic Winter Games and XI Paralympic Winter Games of 2014 in Sochi is considered. Efforts to establish an effective system of laboratory support, the order of work and interaction of diagnostic laboratories involved in diseases control of population during the Olympic Games are analyzed.

[Improving prevention activities of infectious diseases during preparation and holding of the XXII Olympic Winter Games and XI Paralympic Winter Games 2014 in Sochi].

The article presents data on the implementation of a set of preventive activities to ensure sanitation and epidemiological welfare during the XXII Olympic Winter Games and XI Paralympic Winter Games 2014 in Sochi. The importance of monitoring and evaluation of epidemiological risk, as the basis of formation of preventive measures is noticed. The questions of specific, and nonspecific prevention of infectious diseases, especially the work done during the pre-Olympic period are considered.